摘要
OBJECTIVES:We described morphologic characteristics, particularly pulmonary an atomy, and determined the prevalence of definitive end states and their determin ants in children with pulmonary atresia associated with ventricular septal defec t(PAVSD). BACKGROUND: Pulmonary atresia associated with ventricular septal defec t represents a broad morphologic spectrum that greatly influences management and outcomes. METHODS: From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review(n=171) characterized bronchopulmonary s egment arterial supply. RESULTS:A total of 185 patients underwent surgery,and re pair was definitive in 75%. Initial operations included systemic-pulmonary art ery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review,118 patients had sim ple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arterie s(MAPCAs). Overall survival from initial operation was 71%at 10 years. Risk fac tors for death after initial operation included younger age at repair, earlier b irth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteri es, and initial placement of a systemic-pulmonary artery shunt. Competing risks analysis for initially palliated patients predicted that after 10 years, 68%ac hieved complete repair(with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries), 22%died without repair, and 10%remained alive without repair. Reoperations after comple te repair occurred in 38 children(27%), with risk factors including older age a t palliation, MAPCAs, and more segments supplied by collaterals. CONCLUSIONS: Ou tcomes in children with PAVSD have improved over time, and are better in complet ely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.
OBJECTIVES： We described morphologic characteristics, particularly pulmonary anatomy, and determined the prevalence of definitive end states and their determinants in children with pulmonary atresia associated with ventricular septal defect（PAVSD）. BACKGROUND： Pulmonary atresia associated with ventricular septal defect represents a broad morphologic spectrum that greatly influences management and outcomes. METHODS： From 1975 to 2004, 220 children with PAVSD presented to our institution. Blinded angiographic review（n = 171 ） characterized bronchopulmonary segment arterial supply. RESULTS： A total of 185 patients underwent surgery, and repair was definitive in 75%. Initial operations included systemic-pulmonary artery shunt in 57%, complete primary repair in 31%, or right ventricular outflow tract reconstruction in 12%. Based on angiographic review, 118 patients had simple PAVSD and 53 patients had PAVSD with major aortopulmonary collateral arteries （MAPCAs）. Overall survival from initial operation was 71%at 10 years. Risk factors for death after initial operation included younger age at repair, earlier birth cohort, fewer bronchopulmonary segments supplied by native pulmonary arteries, and initial placement of a systemic-pulmonary artery shunt. Competing risks analysis for initially palliated patients predicted that after 10 years, 68% achieved complete repair（with associated factors including later birth cohort and more bronchopulmonary segments supplied by native pulmonary arteries）.
